The two most common forms of pediatric rheumatic disease are juvenile idiopathic arthritis (JIA) and pediatric systemic lupus erythematosus (pSLE). JIA is the most common cause of acquired disability in the US and the fifth most common chronic childhood disease. Children with JIA experience an unpredictable disease course, with periods of improved disease control intermixed with episodes of flare and report poorer HRQOL than healthy peers, with decreased HRQOL, even in the setting of low disease and treatment with biological agents. pSLE is an incurable multisystem autoimmune inflammatory disease affecting an estimated 0.5 in 1,000 children. Children and adolescents with pSLE experience moderate to severe physical disability, especially at times of disease flare, and report chronic low to moderate pain with daily variations in pain intensity. pSLE is also associated with impaired social well-being, increased emotional distress, and decreased overall QOL compared to healthy and rheumatic disease populations.

 

The DCRI CARRA registry began in 2015 with the mission to build a registry of pediatric patients with rheumatic disease to facilitate the conduct of collaborative research to prevent, treat, and cure pediatric rheumatic diseases. Current enrollment in the registry is over 3000 pediatric patients with rheumatic disease.

 

Despite the established importance of patient and parent-proxy reported outcomes in JIA and other pediatric chronic diseases, the lack of a standardized approach to assessing PROs has led to the use of a variety of measures, which has limited the scope of published data. A subset of the pediatric PROMIS measures (physical function upper extremity [PF-upper], physical function mobility [PF-mobility], depression, emotional distress anxiety [ED-anxiety], ED-anger, ED-depression, fatigue, pain interference) have undergone limited validation in JIA and SLE (Morgan, unpublished). The current PEPR Project focuses on multisite validation of additional PROMIS measures as well as further development of meaningfulness and usefulness. PEPR is enrolling approximately 450 CARRA Registry participants (both the patient and their parent proxies) as well as a subset of 75 patients who meet criteria for a pedometry sub-study. The patients and their parent proxies will be followed longitudinally every 6 months for 18 months